2. Definitions
– A group of disorders of the development of movement and posture
causing activity limitations that are attributed to non-progressive
disturbances that occurred in the developing fetal or infant brain.
– Often accompanied by disturbances of sensation, cognition,
communication, perception, and/or behavior and/or a seizure disorder.
3. Definitions
– Variable etiology and severity ranging from minor
incapacitation to total handicap.
– Insult to brain tissue: Fetal to neonatal period up to 3 years.
(R/o progressive disease= diagnosis not made until 5years)
– As the CNS matures, peripheral manifestations of the
central lesions change. Though static, it is not unchanging.
7. Classification
– The classification of cerebral palsy is multiaxial and
includes:
– Type of dysfunction (physiologic)
– Site of dysfunction (topographic)
– The value of physiologic or topographic classification is that
syndromes emerge that are related to patterns of
neurologic deficit.
8. Physiologic Classification
– Cerebral palsy can be divided into two major groups:
– The pyramidal (spastic) type
– The extrapyramidal (non-spastic) type
– There is often marked overlap between the two eg: Patient
with spastic syndrome may have involuntary abnormal
movements.
9. Spastic cerebral palsy
– Commonest form of cerebral palsy (80%).
– Early diagnostic features of neural damage include:
• Persistent neonatal reflexes
• Feeding difficulties
• Persistent cortical thumb after 3 months of age
• Scissoring
• Stretch tendon reflexes are always brisk.
• “clasp knife” muscle tone
– They have variable degrees of mental and visual handicaps, seizures and
behavioral problems.
10. Subclassifications:
– 1. Hemiplagic
– Can be noticed during development: hand dominance.
– Affected side: movement and tone decrease initially before tone and reflexes
typically increase.
• Equinovarus deformity of the foot .
• Child walks tiptoe because of increased tone
• 1/3 of children have seizure disorder; ¼ mental retardation
– →Focal cerebral infarction secondary to intrauterine or
– perinatal thromboembolism is a common cause.
– →Term infants of normal birth weight.
11. – 2. Diplegia –
– Lower limbs > upper limb involvement.
– • 1st indication is when the affected child begins to crawl –
– Uses the arms more, legs act as a rudder.
– • Severe: excessive abduction of the hips makes diapers difficult to apply.
– • Scissoring
– • Walking is delayed. (+) equinovalgus or calcaneovarus deformity of the
foot
• Seizure likelihood: minimal and prognosis for normal
– intellectual development is excellent.
– →PVL in internal capsule
– →Term and preterm infants.
12. – 3. Quadriplegia
• most severe form because of marked motor
– impairment of all extremities and high association with
– mental retardation and seizures
– • Swallowing difficulties : risk of aspiration pneumonia
– • Speech and visual abnormalities are prevalent in this group
– →Infants that are SGA, consistent with a prenatal origin, such as cerebral
dysgenesis or infection. (+)ELBW
13.
14.
15. Extrapyramidal CP
– This form accounts for 10-20% of cases.
– Clinical manifestations include:
– Athetosis
– Choreiform movements
– Dystonia
– Ataxic
– Atonic
– Due to status marmoratus: Lesions in basal ganglia and thalamus.
– Cerebral damage following encephalopathy is one of the causes.
16. • Hypotonic
• Develop increased variable tone with rigidity (lead pipe) over
– several years.
• Feeding difficulties ++ drooling
• Speech affected because oropharyngeal muscles are involved
• Intellect is preserved and seizures are uncommon
– 1. Athetosis – slow, writhing involuntary movement particularly in distal
extremities.
– 2. Chorea – abrupt irregular jerky movement or choreoathetosis.
– 3. Dystonia –repetitive, patterned, twisting, and sustained movements of
the trunk and limbs that may be either slow or rapideg. In jaws and upper
extremities.
17. – 4. Ataxic - Diagnosis of exclusion as all patients with CP may hae
incoordination and disturbed posture.
– 5. Atonic – often absent from contemporary classifications. Severe
hypotonia, for approximately 12 to 18 months and then becomes variable
and paratonic
18. Physiologic Classification
Sign Pyramidal (Spastic) Type Extrapyramidal (Non-spastic) Type
Movement Decreased Disordered, but may be decreased in
rigidity
Oral motor Suprabulbar type (flat facies) Athetotic (grimacing) or suprabulbar
type
Tone changes Consistent and persistent; remain
relatively constant when infant is
asleep or challenged.
Variable; changes with tension,
challenge, or sleep (usually hypotonic
when asleep or quiet)
Tone quality Claps-knife spasticity; sudden give
followed by resistance or vice
versa; more exaggerated with
sudden increase in tendon
stretching
Lead pipe or candle wax rigidity;
sustained resistance throughout
extension or flexion of a limb,
brought out by slow movement.
19. Physiologic Classification
Sign Pyramidal (Spastic) Type Extrapyramidal (Non-spastic) Type
Primitive
reflexes
Present (not noticeable as with
choreoathetoid cerebral palsy)
Present; most evident in this type of
cerebral palsy
Deep tendon
relfexes
Hyperreflexia, predominates, 3+ or
4+ (frequently accompanied by
overflow movement)
Normal or mildly increased 1+ to 3+
Babinski Present Absent, not to be confused with the
athetotic positioning of the toes and
foot (extensor plantar response) due
to the posturing seen with
choreoathetoid movement.
20. Physiologic Classification
Sign Pyramidal (Spastic) Type Extrapyramidal (Non-spastic) Type
Ankle clonus Sustained ankle clonus evident Unsustained ankle clonus; possible
to have some ankle clonus present
Contractures Non-positional contractures Positional contractures such as hip
and knee flexion contractures when
child has been in a wheelchair for
months or years.
21. Topographic Classification
– This classification is limited to the spastic types.
– It is not usually used with extrapyramidal types, because
they have four-limb involvement and are classed by the
nature of the movement disorder.
– The topographic axis includes:
– Hemiplegia/ hemiparesis
– Diplegia
– Quadriplegia/ quadraparesis
22. Topographic Classification
Classification Population Limb Involvement Clinical Manifestations
Hemiplegia/
hemiparesis
(Recognized after 4-6
months)
Term infants of
normal birth
weight
Involvement of either lateral side
of the body.
Upper extremities are more
impaired than lower, and upper
extremity dysfunction usually
brings the child to attention.
• Early hand preference
• Abnormal persistent fisting
• Abnormal posture
• Gait disturbance
• Vascular insults, porencephaly
or cerebral anomalies may be
associated.
Diplegia
(associated with peri-
ventricular leuko-
malacia)
Preterm babies Four-limb involvement with upper
limbs only minimally involved
although there is significant lower
extremity impairment.
The lower limbs are more severely
affected with extension and
adduction posturing, brisk tendon
jerks and contractures.
24. Topographic Classification
Classification Population Limb Involvement Clinical Manifestations
Quadriplegia/
quadraparesis
Term babies Four-limb involvement
with the upper limbs may
be less impaired than
lower, but substantial
functional limitations
exist.
Some researchers
consider quadraplegia a
furtherance of diplegia.
• Opisthotonic posture
• Pseudobulbar palsy
• Feeding difficulties
• Restricted voluntary
movements
• Motor deficits
26. Associated Disorders:
– Cognitive, communicative, and behavioural disturbances
are commonly seen.
– Seizures, sensory loss, and visual and auditory disturbances
influence treatment.
– Mental retardation coexists with cerebral palsy in
approximately 60% of patients.
28. • Urinary Disorders
• Neurologic deterioration
• Sensory defects
• Miscellaneous: Thermoregulation and problems of social
and emotional adjustment are present in many cases.
Dental defects may need to be addressed.
31. Diagnosis
– Made clinically. Evaluation includes:
– Perinatal history
– Detailed neurological & developmental examination
– Assessment of language and learning disabilities.
– Cerebral palsy should be suspected in a child with:
• LBW
• Perinatal insult
• Clinically hypertonic
• Feeding difficulties
• Global developmental delay.
32. Investigations: Laboratory
– Laboratory Studies usually not indicated unless:
1. Clinical history or neuroimaging does not indicate specific structural
abnormality
2. Additional atypical features present
– Potentially helpful tests:
– TFTs, Lactate and pyruvate, Ammonia, Organic and amino acids,
chromosomal analysis, CSF protein (may assist determining asphyxia)
33. Investigations: Imaging
– 1. Early neonatal period: Cranial Ultrasonography
– May show evidence of haemorrhage or hypoxic-ischemic
injury.
– PVL : echodense area that converts to echolucent at 2wks
– 2. Infants: CT
– Congential malformations, haemorrhage , PVL
– 3. MRI: 2-3 weeks of life – older children.
– Normal imaging does not exclude CP.
34. Investigations: Others
– 4. Electroencephalography: Important in diagnosing
seizure disorders with CP and useful in evaluating hypoxic-
ischemic injury.
– 5. Electromyography (EMG) and Nerve conduction studies
– When muscle or nerve disorder is suspected
35. Differential Diagnosis
– Neurodegenrative disorders:
– Failure to thrive, vomiting, seizures are significant symptoms.
– Lab investigations are necessary.
– Hydrocephalus and Subdural effusion:
– Head size is large, fontanelle may bulge and sutures may
separate.
– Brain tumors or SOLs:
– Lesion is progressive and features of increased ICP are evident.
36. Differential Diagnosis
– Muscle disorders:
– Congenital myopathies and muscular dystrophies.
– Distribution of muscle weakness. Hypotonia.
– Creatine phosphokinase may be elevated.
– EMG and muscle biopsy are diagnostic.
– Ataxia-telangiectasia:
– Ataxia may appear before the ocular telangiectasia are evident.
37. Management: Approach
– Individualised, holistic and multidisciplinary.
– The management plan directed to severity, type of neurological deficits
and associated problems.
– Goals of Management: psychological development, communication and
education. Maximising quality of life.
38. Management: Spasticity
– Pharmacotherapy
– - Botulinum Toxin A (BTX)
– Patients with increased muscle tone that interferes with function and likely to
lead to contracture. Older children >18months.
– - Oral Antispastic Drugs:
– Baclofen and tizanidine help to reduce spasticity and athetosis.
– Dantrolene sodium helps in relaxation of skeletal muscles.
39. Management
– Occupational Therapy:
– Feeding and dressing initially, then on to more complex movements.
– Educational:
– The defects of vision, perception, speech and learning are managed by
adequate special education experiences.
– Orthopedic support:
– Tendon, muscle and bony surgeries may be required eg Muscle tendon release.
Neurosurgery: Selective Dorsal Rhizotomy, Stereotactic encephalotomy.
Light weigh splints may be required for tight tendo-Achilles and cortical thumb.
40. – Selective dorsal rhizotomy (SDR) is a surgical procedure
that selectively divides parts of the dorsal lumbosacral
roots of the spinal cord. This interrupts the afferent limb of
the reflex arc on the sensory side, thus reducing spasticity
without causing paralysis. This technique may provide a
small gain in function
– Stereotactic encephalotomy on the basal ganglia (cutting
specific tracts) has been performed in patients with
hyperkinesia or dystonia.
41. Management
– Social:
– The family should be given social and emotional support to
help it to live with the child’s shortcomings.
– Rehabilitation and vocational guidance:
– Parents should help the child to adjust in the society and if
possible to become independent by proper vocational guidance
and rehabilitation.
42. Prevention
– Reduction of prevalence involves:
– Prevention of maternal infection
– Fetal or perinatal insults (especially birth asphyxia)
– Good maternal and neonatal care.
– Early diagnosis, prompt adequate management plans can
reduce the residual neurological and psychosocial
handicaps for the child and his family.
43.
44.
45. References
– Cerebral Palsy Alliance Australia. (2015). Types of Cerebral Palsy. [Online Video]. 29 July
2015. Available from: https://www.youtube.com/watch?v=cOfUGUNxEqU. [Accessed:
19 February 2018]
– Cerebral Palsy Information - Types, Pictures, Causes, Treatment.. 2018. Cerebral Palsy
Information - Types, Pictures, Causes, Treatment.. [ONLINE] Available
at: https://howshealth.com/cerebral-palsy-information/. [Accessed 19 February 2018]
– Cerebral Palsy: Practice Essentials, Background, Anatomy. 2018. Cerebral Palsy: Practice
Essentials, Background, Anatomy. [ONLINE] Available at:
https://emedicine.medscape.com/article/1179555-overview#a2. [Accessed 19
February 2018].
– Ghai. O.P., 2013. Essential Pediatrics. CBS Publishers & Distributors Pvt Ltd
– Miller, G, 2009. Epidemiology and etiology of cerebral palsy. UptoDate, [Online]. 1, 1-
39. Available at:
http://cursoenarm.net/UPTODATE/contents/mobipreview.htm?19/59/20400?source=
HISTORY [Accessed 16 February 2018].
– Oski. F.A., 1994. Principles and Practice of Pediatrics. J. B Lippincott Company
Editor's Notes
A disorder of muscle tone and posture due to static motor encephalopathy
Dynamic manifestation usually includes mental retardation and seizure
May or may not involve organs of special sensation.
Results from any insult to developing brain.
Although a static encephalopathy, cerebral palsy is not unchanging.
Perinatal asphyxia (though not guided by low APGAR)
Neonatal Encephalopathy (hypoxia-ischemia)
Congenital abnormalities(structural)
Genetic susceptibility (consanguinity)
Multiple births (increased risk of LBW, anomalies, cord entanglement)
Stroke in perinatal period.
Periventricular leukomalacia (PVL)
Intracranial haemorrhage
Intrauterine infection (TORCH and chorioamnionitis)
Prematurity
MgSO4 (potentially neuroprotective)
E.g.: hemiplegia may be associated with growth arrest, a visual field defect (e.g. homonymous hemianopia), sensory impairment, and seizures.
The pyramidal pathways (corticospinal and some corticobulbar tracts) may directly innervate motor neurons of the spinal cord or brainstem (anterior (ventral) horn cells or certain cranial nerve nuclei), whereas the extrapyramidal system centers on the modulation and regulation (indirect control) of anterior (ventral) horn cells.
Extrapyramidal tracts are chiefly found in the reticular formation of the pons and medulla, and target neurons in the spinal cord involved in reflexes, locomotion, complex movements, and postural control. These tracts are in turn modulated by various parts of the central nervous system, including the nigrostriatal pathway, the basal ganglia, the cerebellum, the vestibular nuclei, and different sensory areas of the cerebral cortex. All of these regulatory components can be considered part of the extrapyramidal system, in that they modulate motor activity without directly innervating motor neurons.
Abnormally persistent neonatal reflexes
Feeding difficulties
Persistent cortical thumb after 3 months of age and a firm grasp.
Scissoring: On vertical suspension due to adductor spasm with an extensor posture and infant does not flex his knees or thigh.
Stretch tendon reflexes are always brisk.
“clasp knife” (velocity-dependent - present @ extremes of movement) muscle tone
Periventricular leukomalacia occurs because of ischemia induced injury to oligodendrocytes in the periventricular area of the developing brain. Periventricular leukomalacia is a white matter lesion in premature infants that results from hypotension, ischemia, and coagulation necrosis at the border or watershed zones of deep penetrating arteries of the middle cerebral artery.
Affected patients have variable degrees of flexion at the elbows and knees and flexion, adduction, and internal rotation of the hips. They may have an equinovalgus or calcaneovarus deformity of the foot. Extension of the fingers, abduction of the thumb, extension of the wrist, and supination of the forearm may be limited
Hemiplagic Posture:
The arm is adducted at the shoulder and flexed at the elbow, the forearm is pronated, and the wrist and fingers are flexed with the hand closed. The hip is partially flexed and adducted, and the knee and ankle are flexed because of increased tone in the hamstring and plantar flexor muscles
Diplegic Posture:
Affected patients have variable degrees of flexion at the elbows and knees and flexion, adduction, and internal rotation of the hips. They may have an equinovalgus or calcaneovarus deformity of the foot. Extension of the fingers, abduction of the thumb, extension of the wrist, and supination of the forearm may be limited.
Athetosis - a condition in which abnormal muscle contractions cause involuntary writhing movements. It affects some people with cerebral palsy, impairing speech and use of the hands.
Choreiform movements - repetitive and rapid, jerky, involuntary movement that appears to be well-coordinated
Dystonia - a state of abnormal muscle tone resulting in muscular spasm and abnormal posture, typically due to neurological disease or a side effect of drug therapy.
paratonic (ie, with passive movement of a joint, resistance increases proportionally to the amount of pressure applied).
Choreoathetosis is the occurrence of involuntary movements in a combination of chorea (irregular migrating contractions) and athetosis (twisting and writhing). It is caused by many different diseases and agents.
Porencephaly is an extremely rare cephalic disorder involving encephalomalacia. It is a neurological disorder of the central nervous system characterized by cysts or cavities within the cerebral hemisphere.
Encephalomalacia is the softening or loss of brain tissue after cerebral infarction, cerebral ischemia, infection, craniocerebral trauma, or other injury.
Periventricular leukomalacia (PVL) is a form of white-matter brain injury, characterized by the necrosis (more often coagulation) of white matter near the lateral ventricles. It can affect newborns and (less commonly) fetuses; premature infants are at the greatest risk of the disorder.
Diplegia: Paraplegia is reserved for spinal and LMN dysfunctions, such as myelodysplasia.
Cerebral palsy rarely occurs without associated deficits. The diagnosis of cerebral palsy alone is not sufficient.
In spastic forms, mental retardation is more frequent and more severe in proportion to the number of limbs involved. However, motor impairment does not always mean mental retardation.
Mental Retardation: approx. 60%. Correlates with degree of motor impairment. Variable.
Psychiatric Disorders: Behavourial and emotional disorders common. OCD, emotional lability, poor attention and vigilance.
Seizure disorder: 30-50%. MR = seizures more common.
Visual Disorders: Amblyopia, visual field defects, strabismus, low visual acuity.
Speech impairment: apshia, dysarthria. ++hearing impairment.
Growth Failure: often due to poor nutrition
Orthopedic disorders: sublaxation, dislocation, dyplasia of the hip. ++Osteopenia (lack of mobility, poor nutrition) – fractures
Urinary Disorders: One third may have voiding symptoms.
Neurologic deterioration: Possibility for neurologic symptoms to worsen with age.
Sensory defects: Astereognosis and spatial disorientation are seen in 1/3 or the patients. Deafness (esp in patients with kernicterus)
Miscellaneous: Inadequate thermoregulation and problems of social and emotional adjustment are present in many cases. Dental defects may need to be addressed.
Tests mostly for inborn errors of metabolism.
Identification of associated deficits is important for appropriate physiotherapy and occupational therapy.
.
Tizanidine is a short-acting muscle relaxer. It works by blocking nerve impulses (pain sensations) that are sent to your brain.Tizanidine is used to treat spasticity by temporarily relaxing muscle tone.
Dantrolene sodium reduces muscle contraction by inhibiting release of calcium ions from the sarcoplasmic reticulum. Side effects, which are common, include weakness, drowsiness, lethargy, paresthesias, nausea, and diarrhea
Baclofen an analog of GABA, binds to the GABA-B receptors on the terminals of primary afferent fibers. Activation of these receptors inhibits the influx of calcium ions into presynaptic terminals and suppresses release of excitatory neurotransmitters. This inhibits monosynaptic and polysynaptic reflexes and reduces gamma efferent activity. Side effects include confusion, sedation, hypotonia, ataxia, paresthesias, and nausea. Seizures and hallucination may occur if the drug is discontinued abruptly .
Selective dorsal rhizotomy (SDR) is a surgical procedure that selectively divides parts of the dorsal lumbosacral roots of the spinal cord. This interrupts the afferent limb of the reflex arc on the sensory side, thus reducing spasticity without causing paralysis. This technique may provide a small gain in function
Stereotactic encephalotomy on the basal ganglia (cutting specific tracts) has been performed in patients with hyperkinesia or dystonia.
